Histological Type-Specific Behavior in Sarcomas: Analysis of Head and Neck Cancer Registry of Japan.
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OBJECTIVE: The goal of this study was to better understand the epidemiology, clinical characteristics, and treatment outcomes of head and neck sarcomas using real-world data from Japan. METHODS: Using the Japanese Head and Neck Cancer Registry, we identified 438 patients who were pathologically diagnosed with head and neck sarcoma between 2011 and 2020. We compared epidemiological, clinical, and prognostic data for the different histological types of sarcoma. RESULTS: The top five most common histopathological types were rhabdomyosarcoma, osteosarcoma, chondrosarcoma, leiomyosarcoma, and liposarcoma. The most common primary sites were the nasal cavity and paranasal sinuses. The overall cohort had clinical, pathologic, and occult lymph node positivity rate (OLR) of 18%, 15%, and 2%, respectively. Clinical lymph node positivity was highest in rhabdomyosarcoma (46%), followed by angiosarcoma (26%), with leiomyosarcoma, chondrosarcoma, and liposarcoma accounting for less than 10%. The 3-year overall survival rate was 74.8% among the 147 patients with available prognostic information. The histological type with the lowest 3-year overall survival was undifferentiated pleomorphic sarcoma (54%), followed by leiomyosarcoma (65%), and angiosarcoma (69%). In six of seven histological types, the 3-year locoregional-free survival rate was less than 70%. CONCLUSION: Sarcomas of the head and neck most commonly occur in the nasal cavity and paranasal sinuses. The prevalent histological type depended on the primary site. Rhabdomyosarcoma and angiosarcoma are histological types with a high risk of cervical metastasis. In the head and neck region, there are numerous histological types many of which lack local control. LEVEL OF EVIDENCE: 3 Laryngoscope, 135:2403-2410, 2025.